The efficacy of topiramate in adult refractory status epilepticus: experience of a tertiary care center.

Refractory status epilepticus (RSE) occurs in patients with SE when they fail to respond to traditional medical therapy. Because there are very few case reports of topiramate (TPM) treatment of RSE in adult patients, we examined our experience with TPM with regard to its safety and efficacy in seizure termination in RSE in an adult patient population. We report a retrospective review of 35 adult patients with RSE who were treated with TPM in addition to other antiepileptic drugs (AEDs) between 2003 and 2010. After failure of initial treatments of benzodiazepines and weight-based intravenous loading doses of standard AEDs, TPM tablets were crushed and administered via nasogastric tube. Data were collected on age, gender, history of epilepsy, etiology of RSE, daily dose of TPM, co-therapeutic agents, treatment response, and disposition. Following initiation of TPM use and discontinuation of continuous intravenous anesthetics with no additional AEDs administered, cumulative cessation of RSE in patients was 4/35 (11%) at one day, 10/35 (29%) at two days, and 14/35 (40%) at three days. However, when including all patients and comparing the two patient groups in which RSE was or was not terminated within three days of initiating TPM as the last or not last AED given, there was no significant difference. Time to TPM response was not associated with the type of seizures, etiology of SE, or whether there was a history of epilepsy. There were no documented side effects or complications of therapy with TPM. This study provides support for the use of TPM as an adjunctive agent in the treatment of RSE.

Neurosarcoidosis mimicry of multiple sclerosis: clinical, laboratory, and imaging characteristics.

OBJECTIVE: To characterize the clinical and laboratory features of neurosarcoidosis (NS), presenting with findings consistent with multiple sclerosis (MS). METHODS: Retrospective chart review of our entire NS database was undertaken. Patients initially diagnosed with MS but who were subsequently diagnosed as having both systemic and neurologic sarcoidosis years later were selected for more detailed review. RESULTS: Seven patients were identified who were diagnosed with MS (although only 4 of these met McDonald criteria for MS during chart review) and 1 patient with optic neuritis who had a diagnosis of likely MS. These patients maintained the diagnosis of MS for a mean of 107 months (median 50 mo, range 23 to 262 mo) before the diagnosis was changed to NS, concomitant with the discovery of biopsy-proven systemic sarcoidosis in 4 cases. Neurologic manifestations included relapsing-remitting optic neuritis, myelopathy, dystonic spasms, sensory abnormalities, paraparesis, and hemiparesis. Patients appeared to improve or stabilize by treatment with corticosteroids or alternative immunosuppressants. MRIs demonstrated rounded or ovoid periventricular white matter changes typical for MS. CONCLUSION: Patients with NS are frequently diagnosed initially with MS because of a considerable overlap of clinical and laboratory features. However, due to the relative rarity of NS, a misdiagnosis of NS as MS occurred only infrequently in our MS clinic.

Aggressive therapy for neurosarcoidosis: long-term follow-up of 48 treated patients.

BACKGROUND: Neurosarcoidosis (NS) is a relatively rare neurologic disorder for which no accepted treatment guidelines are available. Treatment with corticosteroids has been described as the primary means of controlling progressive symptoms. However, some physicians have recently advocated early intervention with alternative immunosuppressive therapies in patients who present with disabling symptoms. OBJECTIVE: To investigate our experience during the last decade regarding alternative immunosuppressive treatments, including corticosteroids and alternative therapies, in patients with NS. DESIGN: Observational, retrospective, consecutive case series with longitudinal follow-up. SETTING: Allegheny Neurological Clinic. Patients Seventy-eight patients with sarcoidosis were evaluated and classified as having possible, probable, or definite NS according to accepted criteria. Five cases of isolated NS were also included. MAIN OUTCOME MEASURES: Patients with probable, definite, or isolated NS were scored before treatments and at final follow-up using estimated modified Rankin scores and the Disease Steps in Multiple Sclerosis scales. RESULTS: Forty-three patients were categorized as having either definite or probable NS according to accepted criteria and an additional 5 as having isolated NS. Thirty patients were categorized as having possible NS and were not included in the analysis of treatment response. Patients had a mean +/- SD number of visits of 7.2 +/- 6.4 and were followed up for a mean +/- SD of 44.1 +/- 43.6 months. Twenty patients were treated with pulse and/or maintenance corticosteroids alone. Twenty-six patients were treated with alternative immunosuppressive medications, with 23 of them receiving these medications at the time of diagnosis or within 6 months of the diagnosis of NS. Of the patients treated with alternative immunosuppressive therapies, 18 (69%) improved, 4 (15%) remained stable, and 4 (15%) worsened (including 1 death). Of the patients treated with corticosteroids alone, 7 (35%) improved, 11 (55%) remained stable, and 2 (10%) worsened. Two patients received no treatment. CONCLUSIONS: Approximately half of all patients with NS seen at our clinic were believed to have disabling disease and to be at high risk for disease progression. These high-risk patients were treated with corticosteroids plus alternative immunosuppressive therapy, and favorable outcomes were obtained in almost all patients. Toxic effects related to treatments were minimal.

The Th1 versus Th2 cytokine profile in cerebrospinal fluid after severe traumatic brain injury in infants and children.

OBJECTIVE: To further characterize the Th1 (proinflammatory) vs. the Th2 (antiinflammatory) cytokine profile after severe traumatic brain injury (TBI) by quantifying the ventricular cerebrospinal fluid concentrations of Th1 cytokines (interleukin [IL]-2 and IL-12) and Th2 cytokines (IL-6 and IL-12) in infants and children. DESIGN: Retrospective study. SETTING: University children's hospital. PATIENTS: Twenty-four children hospitalized with severe TBI (admission Glasgow Coma Scale score, <13) and 12 controls with negative diagnostic lumbar punctures. INTERVENTIONS: All TBI patients received standard neurointensive care, including the placement of an intraventricular catheter for continuous drainage of cerebrospinal fluid. MEASUREMENTS AND MAIN RESULTS: Ventricular cerebrospinal fluid samples (n = 105) were collected for as long as the catheters were in place (between 4 hrs and 222 hrs after TBI). Cerebrospinal fluid samples were analyzed for IL-2, IL-4, IL-6, and IL-12 concentrations by enzyme-linked immunoassay. Peak and mean IL-6 (335.7 +/- 41.4 pg/mL and 259.5 +/- 37.6 pg/mL, respectively) and IL-12 (11.4 +/- 2.2 pg/mL and 4.3 +/- 0.8 pg/mL, respectively) concentrations were increased (p <.05) in children after TBI vs. controls (2.3 +/- 0.7 pg/mL and 1.0 +/- 0.5 pg/mL) for IL-6 and IL-12, respectively. In contrast, peak and mean IL-2 and IL-4 concentrations were not increased in TBI children vs. controls. Increases in the cerebrospinal fluid concentration of IL-6 were significantly associated with admission Glasgow Coma Scale score of